An autopsy heart from a 4-year-old boy with a premortem history of cyanotic congenital heart disease displays right ventricular hyperplasia, pulmonary stenosis, ventricular septal defect, and dextroposition of the aorta. What term best defines this disorder?
- (A) anomalous pulmonary venous drainage
- (B) dextrocardia
- © ebstein malformation
- (D) transposition of the great arteries
- (E) Tetralogy of Fallot
(E) Tetralogy of Fallot includes right ventricular hyperplasia, pulmonary stenosis, ventricular septal defect, and dextroposition of the aorta. It is a cyanotic congenital heart disorder with a dismal prognosis, unless surgically corrected. Anomalous pulmonary venous drainage (choice A) occurs when there is aberrant drainage of the pulmonary veins into the left atrium. Dextrocardia (choice B) is a mirror image inversion of the heart. Ebstein malformation (choice C)is a congenital abnormality of the tricuspid valve and right ventricle. Transposition of
the great arteries (choice D) is a congenital heart disease defined as the aorta arising from the right ventricle and the pulmonary artery originating from the left ventricle.